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Aquaporin 4

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Title: Aquaporin 4  
Author: World Heritage Encyclopedia
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Subject: List of MeSH codes (D12.776.543), Glymphatic system, Membrane biology, Integral membrane proteins, Chronic relapsing inflammatory optic neuropathy
Publisher: World Heritage Encyclopedia

Aquaporin 4

Aquaporin 4
Double layered 2D crystal structure of aquaporin-4 (AQP4M23) at 3.2 a resolution by electron crystallography.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols  ; HMIWC2; MIWC
External IDs IUPHAR: ChEMBL: GeneCards:
Species Human Mouse
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

Aquaporin 4 also known as AQP4 is a protein which in humans is encoded by the AQP4 gene.[1] AQP4 belongs to the aquaporin family of integral membrane proteins that conduct water through the cell membrane.


  • Function 1
  • Pathology 2
  • See also 3
  • References 4
  • Further reading 5
  • External links 6


AQP4 is constitutively expressed in the basolateral cell membrane of principal collecting duct cells in the kidney and provide a pathway for water to exit these cells.[2]

AQP4 is also expressed in astrocytes and is upregulated by direct insult to the central nervous system.[3]


Aquaporin-4 is the primary autoimmune target in neuromyelitis optica,[4][5] in which AQP4-specific IgG antibodies target astrocytic AQP4, resulting in complement activation and inflammation.[6]

See also


  1. ^ Jung JS, Bhat RV, Preston GM, Guggino WB, Baraban JM, Agre P (December 1994). "Molecular characterization of an aquaporin cDNA from brain: candidate osmoreceptor and regulator of water balance". Proceedings of the National Academy of Sciences of the United States of America 91 (26): 13052–6.  
  2. ^ Agre P, Nielsen S (1996). "The aquaporin family of water channels in kidney". Nephrologie 17 (7): 409–15.  
  3. ^ Nagelhus EA, Mathiisen TM, Ottersen OP (2004). "Aquaporin-4 in the central nervous system: cellular and subcellular distribution and coexpression with KIR4.1". Neuroscience 129 (4): 905–13.  
  4. ^ Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR (August 2005). "IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel". J. Exp. Med. 202 (4): 473–7.  
  5. ^ Bradl M, Lassmann DH (September 2008). "Anti-aquaporin-4 antibodies in neuromyelitis optica: how to prove their pathogenetic relevance?" (PDF). International MS Journal / MS Forum 15 (3): 75–8.  
  6. ^ Papadopoulos MC, Bennett JL, Verkman AS (Aug 12, 2014). "Treatment of neuromyelitis optica: state-of-the-art and emerging therapies.". Nature reviews. Neurology 10: 493–506.  

Further reading

  • Strand L, Moe SE, Solbu TT, Vaadal M, Holen T (2009). "Roles of aquaporin-4 isoforms and amino acids in square array assembly". Biochemistry 48 (25): 5785–93.  
  • Wu H, Zhang Z, Li Y, Zhao R, Li H, Song Y, Qi J, Wang J. (2010). "Time course of upregulation of inflammatory mediators in the hemorrhagic brain in rats: correlation with brain edema". Neurochem Int. 57 (3): 248–53.  
  • Goodyear MJ, Crewther SG, Junghans BM (2009). "A role for aquaporin-4 in fluid regulation in the inner retina". Vis. Neurosci. 26 (2): 159–65.  
  • Matsushita T, Matsuoka T, Isobe N, Kawano Y, Minohara M, Shi N, Nishimura Y, Ochi H, Kira J (2009). "Association of the HLA-DPB1*0501 allele with anti-aquaporin-4 antibody positivity in Japanese patients with idiopathic central nervous system demyelinating disorders". Tissue Antigens 73 (2): 171–6.  
  • Rubino E, Rainero I, Vaula G, Crasto F, Gravante E, Negro E, Brega F, Gallone S, Pinessi L (2009). "Investigating the genetic role of aquaporin4 gene in migraine". The journal of headache and pain 10 (2): 111–4.  
  • Benarroch EE (2007). "Aquaporin-4, homeostasis, and neurologic disease". Neurology 69 (24): 2266–8.  
  • Ho JD, Yeh R, Sandstrom A, Chorny I, Harries WE, Robbins RA, Miercke LJ, Stroud RM (2009). "Crystal structure of human aquaporin 4 at 1.8 A and its mechanism of conductance". Proc. Natl. Acad. Sci. U.S.A. 106 (18): 7437–42.  
  • Assereto S, Mastrototaro M, Stringara S, Gazzerro E, Broda P, Nicchia GP, Svelto M, Bruno C, Nigro V, Lisanti MP, Frigeri A, Minetti C (2008). "Aquaporin-4 expression is severely reduced in human sarcoglycanopathies and dysferlinopathies". Cell Cycle 7 (14): 2199–207.  
  • Dibas A, Yang MH, He S, Bobich J, Yorio T (2008). "Changes in ocular aquaporin-4 (AQP4) expression following retinal injury". Mol. Vis. 14: 1770–83.  
  • Sorani MD, Zador Z, Hurowitz E, Yan D, Giacomini KM, Manley GT (2008). "Novel variants in human Aquaporin-4 reduce cellular water permeability". Hum. Mol. Genet. 17 (15): 2379–89.  
  • Ng WH, Hy JW, Tan WL, Liew D, Lim T, Ang BT, Ng I (2009). "Aquaporin-4 expression is increased in edematous meningiomas". Journal of Clinical Neuroscience 16 (3): 441–3.  
  • Nishiyama S, Ito T, Misu T, Takahashi T, Kikuchi A, Suzuki N, Jin K, Aoki M, Fujihara K, Itoyama Y (2009). "A case of NMO seropositive for aquaporin-4 antibody more than 10 years before onset". Neurology 72 (22): 1960–1.  
  • Misu T, Fujihara K, Itoyama Y (May 2008). "Neuromyelitis optica and anti-aquaporin 4 antibody – an overview". Brain Nerve (in Japanese) 60 (5): 527–37.  
  • Dibas AI, Mia AJ, Yorio T (1998). "Aquaporins (water channels): role in vasopressin-activated water transport". Proc. Soc. Exp. Biol. Med. 219 (3): 183–99.  
  • Doi H, Matsushita T, Isobe N, Matsuoka T, Minohara M, Ochi H, Kira JI (2009). "Hypercomplementemia at relapse in patients with anti-aquaporin-4 antibody". Mult. Scler. 15 (3): 304–10.  
  • Pittock SJ, Lennon VA (2008). "Aquaporin-4 autoantibodies in a paraneoplastic context". Arch. Neurol. 65 (5): 629–32.  
  • Matsushita T, Isobe N, Matsuoka T, Shi N, Kawano Y, Wu XM, Yoshiura T, Nakao Y, Ishizu T, Kira JI (2009). "Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese". Mult. Scler. 15 (7): 834–47.  
  • Graber DJ, Levy M, Kerr D, Wade WF (2008). "Neuromyelitis optica pathogenesis and aquaporin 4". Journal of neuroinflammation 5: 22.  
  • Baba T, Nakashima I, Kanbayashi T, Konno M, Takahashi T, Fujihara K, Misu T, Takeda A, Shiga Y, Ogawa H, Itoyama Y (2009). "Narcolepsy as an initial manifestation of neuromyelitis optica with anti-aquaporin-4 antibody". J. Neurol. 256 (2): 287–8.  
  • Xu H, Zhang Y, Wei W, Shen L, Wu W (2009). "Differential expression of aquaporin-4 in human gastric normal and cancer tissues". Gastroenterol. Clin. Biol. 33 (1 Pt 1): 72–6.  
  • Kadohira I, Abe Y, Nuriya M, Sano K, Tsuji S, Arimitsu T, Yoshimura Y, Yasui M (2008). "Phosphorylation in the C-terminal domain of Aquaporin-4 is required for Golgi transition in primary cultured astrocytes". Biochem. Biophys. Res. Commun. 377 (2): 463–8.  

External links

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